AB0761 MULTICENTRIC SPANISH ANALYSIS OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS PATIENTS TREATED WITH MEPOLIZUMAB
Authors: F. Rubiño, R. Garcia-Vicuna, E. F. Vicente-Rabaneda, E. Rodríguez Almaraz, N. Lozano Rivas, M. Freire González, A. Olive, A. Nack, J. Narváez, P. Moya, E. Uriarte Isacelaya, N. Alcorta Lorenzo, J. L. Marenco, C. Ramos Giráldez, V. Pinillos, R. Blanco, F. Benavides-Villanueva, M. P. Martínez-Vidal, I. Rua-Figueroa
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> inophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is an ANCA-associated vasculitis which is mainly mediated by eosinophils. Mepolizumab (MPZ) is an anti-interleukin-5 monoclonal antibody that reduces the total number of eosinophils in peripheral blood and tissues, and its use has been approved in EGPA. <h3>Objectives</h3> To analyze the effectiveness and safety of MPZ in EGPA in a real life multicenter Spanish cohort. <h3>Methods</h3> We retrospectively analyzed the epidemiological data, comorbidities, clinical evolution and adverse effects of patients who have received MPZ in 13 Rheumatology Spanish services, through electronic medical records up to November 2022. <h3>Results</h3> A total of 27 patients with EGPA treated with MPZ have been analyzed. Median age at diagnosis was 53 years old, fifteen were men (55.6%), 21(77.8%) were Caucasian, 5 (18.5%) Arabic and 1 Hispanic. At diagnosis, 3 (11.1%) patients had arterial hypertension and 4 dyslipidemia (14.8%); ten patients were or had been smokers (37%). A total of 14 (51.8%) patients were ANCA positive. The Five Factor Score (FFS) was calculated just in 20 patients, and from them, 14 had a 0 result (70%) and 6 a 1 result (30%); the mean Birmingham Vasculitis Activity Score (BVAS) at diagnosis was 15. EULAR severity index was 0 in 2 patients (7.7%), 1 in 10 patients (38.5%), 2 in 9 (34.6%) and 3 in 5 patients (19.2%). Up to 23 patients (85.2%) had rhinosinusal symptoms and 19 (70.4%), had lung involvement of which 16 patients had interstitial lung disease. Sixteen (59.3%) patients had general symptoms 14 (51.9%) skin involvement and 11 (40.7%) peripheral nervous system involvement. Nine (33.3%) patients had musculoskeletal symptoms, 5 eye involvement and 5 cardiac involvement (18.5%). Regarding treatments, the mean starting dose of daily prednisone was 45 mg, 9 patients received intravenous methylprednisolone pulses (33.3%) and 16 (59.3%) required an immunosuppressant for induction, of which in 11 it was cyclophosphamide. The mean disease duration of the EGPA when MPZ was started was 51 months, and the mean follow-up during the treatment with MPZ was 16 months. The mean Birmingham Vasculitis Activity Score (BVAS) before MPZ was 9, after one month of MPZ, it dropped down to 5.4, and three months later to 4.6. The mean C reactive protein (CRP) before treatment with MPZ was 19.9 mg/L, and three months later, it was 3.3 mg/L. Before MPZ, the patients had a mean eosinophils number per microliter of 753, and after three months of treatment, it was 59 eos/µL. The mean Vasculitis Damage Index (VDI) before treatment with MPZ was 4.5 and after treatment with mepolizumab, it worsened only in 3 patients (11.1%). Twenty patients achieved a 50% clinical response according to global physician criteria (74.1%) and 12 achieved clinical remission (44.4%). The mean final prednisone dose was 2.5 mg/d. No patients had serious infections during treatment, one developed a neoplasm, and one of them withdrew MPZ due to musculoskeletal pain, switching to benralizumab (anti-IL5-receptor). Only one patient died from causes unrelated to the drug or the disease. <h3>Conclusion</h3> Our data about the use of MPZ in EGPA in real world practice suggest that is a quite effective and safe drug, allowing to save corticosteroids. Moreover, it seems to prevent the progression of the damage associated with the disease. <h3>References</h3> [1]Wechsler, M. E., Akuthota, P., Jayne, D., Khoury, P., Klion, A., Langford, C. A., et al. (2017). Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. <i>The New England journal of medicine</i>, <i>376</i>(20), 1921–1932. [2]Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016; 75:1583–94 [3]Furuta, S., Iwamoto, T., &amp; Nakajima, H. (2019). Update on eosinophilic granulomatosis with polyangiitis. <i>Allergology International: Official Journal of the Japanese Society of Allergology</i>, <i>68</i>(4), 430–436. https://doi.org/10.1016/j.alit.2019.06.004 <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.